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Recurrent Hemophagocytic Lymphohistiocytosis

MedGen UID:
1661552
•Concept ID:
C4727151
•
Disease or Syndrome

Definition

The reemergence of hemophagocytic lymphohistiocytosis after a period of remission. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRecurrent Hemophagocytic Lymphohistiocytosis

Professional guidelines

PubMed

Consensus-Based Guidelines for the Recognition, Diagnosis, and Management of Hemophagocytic Lymphohistiocytosis in Critically Ill Children and Adults.
Hines MR, von Bahr Greenwood T, Beutel G, Beutel K, Hays JA, Horne A, Janka G, Jordan MB, van Laar JAM, Lachmann G, Lehmberg K, Machowicz R, Miettunen P, La Rosée P, Shakoory B, Zinter MS, Henter JI
Crit Care Med 2022 May 1;50(5):860-872. Epub 2021 Oct 5 doi: 10.1097/CCM.0000000000005361. PMID: 34605776
Nivolumab treatment of relapsed/refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in adults.
Liu P, Pan X, Chen C, Niu T, Shuai X, Wang J, Chen X, Liu J, Guo Y, Xie L, Wu Y, Liu Y, Liu T
Blood 2020 Mar 12;135(11):826-833. doi: 10.1182/blood.2019003886. PMID: 31914172
Treatment of hemophagocytic lymphohistiocytosis.
Wang Y, Wang Z
Curr Opin Hematol 2017 Jan;24(1):54-58. doi: 10.1097/MOH.0000000000000302. PMID: 27755125

Recent clinical studies

Etiology

Haploidentical Hematopoietic Stem Cell Transplantation for XIAP Deficiency: a Single-Center Report.
Yang J, Zhu GH, Wang B, Zhang R, Jia CG, Yan Y, Ma HH, Qin MQ
J Clin Immunol 2020 Aug;40(6):893-900. Epub 2020 Jul 5 doi: 10.1007/s10875-020-00795-6. PMID: 32627096
Familial hemophagocytic lymphohistiocytosis in a girl with a novel homozygous mutation of STX11: A case report.
Guo X, Jiang M, Tang X, Li Q
Medicine (Baltimore) 2019 Nov;98(48):e18107. doi: 10.1097/MD.0000000000018107. PMID: 31770233Free PMC Article
Treosulfan-based conditioning and hematopoietic cell transplantation for nonmalignant diseases: a prospective multicenter trial.
Burroughs LM, Nemecek ER, Torgerson TR, Storer BE, Talano JA, Domm J, Giller RH, Shimamura A, Delaney C, Skoda-Smith S, Thakar MS, Baker KS, Rawlings DJ, Englund JA, Flowers ME, Deeg HJ, Storb R, Woolfrey AE
Biol Blood Marrow Transplant 2014 Dec;20(12):1996-2003. Epub 2014 Sep 6 doi: 10.1016/j.bbmt.2014.08.020. PMID: 25196857Free PMC Article

Diagnosis

Mutations at the C-terminus of CDC42 cause distinct hematopoietic and autoinflammatory disorders.
Coppola S, Insalaco A, Zara E, Di Rocco M, Marafon DP, Spadaro F, Pannone L, Farina L, Pasquini L, Martinelli S, De Benedetti F, Tartaglia M
J Allergy Clin Immunol 2022 Jul;150(1):223-228. Epub 2022 Feb 12 doi: 10.1016/j.jaci.2022.01.024. PMID: 35157921
Identification of Germline Non-coding Deletions in XIAP Gene Causing XIAP Deficiency Reveals a Key Promoter Sequence.
Sbihi Z, Tanita K, Bachelet C, Bole C, Jabot-Hanin F, Tores F, Le Loch M, Khodr R, Hoshino A, Lenoir C, Oleastro M, Villa M, Spossito L, Prieto E, Danielian S, Brunet E, Picard C, Taga T, Abdrabou SSMA, Isoda T, Yamada M, Palma A, Kanegane H, Latour S
J Clin Immunol 2022 Apr;42(3):559-571. Epub 2022 Jan 9 doi: 10.1007/s10875-021-01188-z. PMID: 35000057
Absent X-linked inhibitor of apoptosis protein expression in T cell blasts and causal mutations including non-coding deletion.
Abdrabou SSMA, Toita N, Ichihara S, Tozawa Y, Takahashi M, Fujiwara SI, Ashida T, Ohara O, Ariga T, Manabe A, Konno M, Yamada M
Pediatr Int 2022 Jan;64(1):e14892. doi: 10.1111/ped.14892. PMID: 34145698
Haploidentical Hematopoietic Stem Cell Transplantation for XIAP Deficiency: a Single-Center Report.
Yang J, Zhu GH, Wang B, Zhang R, Jia CG, Yan Y, Ma HH, Qin MQ
J Clin Immunol 2020 Aug;40(6):893-900. Epub 2020 Jul 5 doi: 10.1007/s10875-020-00795-6. PMID: 32627096
Hematopoietic Stem Cell Transplantation for XIAP Deficiency in Japan.
Ono S, Okano T, Hoshino A, Yanagimachi M, Hamamoto K, Nakazawa Y, Imamura T, Onuma M, Niizuma H, Sasahara Y, Tsujimoto H, Wada T, Kunisaki R, Takagi M, Imai K, Morio T, Kanegane H
J Clin Immunol 2017 Jan;37(1):85-91. Epub 2016 Nov 4 doi: 10.1007/s10875-016-0348-4. PMID: 27815752Free PMC Article

Therapy

Haploidentical Hematopoietic Stem Cell Transplantation for XIAP Deficiency: a Single-Center Report.
Yang J, Zhu GH, Wang B, Zhang R, Jia CG, Yan Y, Ma HH, Qin MQ
J Clin Immunol 2020 Aug;40(6):893-900. Epub 2020 Jul 5 doi: 10.1007/s10875-020-00795-6. PMID: 32627096
Hematopoietic Stem Cell Transplantation for XIAP Deficiency in Japan.
Ono S, Okano T, Hoshino A, Yanagimachi M, Hamamoto K, Nakazawa Y, Imamura T, Onuma M, Niizuma H, Sasahara Y, Tsujimoto H, Wada T, Kunisaki R, Takagi M, Imai K, Morio T, Kanegane H
J Clin Immunol 2017 Jan;37(1):85-91. Epub 2016 Nov 4 doi: 10.1007/s10875-016-0348-4. PMID: 27815752Free PMC Article
Treosulfan-based conditioning and hematopoietic cell transplantation for nonmalignant diseases: a prospective multicenter trial.
Burroughs LM, Nemecek ER, Torgerson TR, Storer BE, Talano JA, Domm J, Giller RH, Shimamura A, Delaney C, Skoda-Smith S, Thakar MS, Baker KS, Rawlings DJ, Englund JA, Flowers ME, Deeg HJ, Storb R, Woolfrey AE
Biol Blood Marrow Transplant 2014 Dec;20(12):1996-2003. Epub 2014 Sep 6 doi: 10.1016/j.bbmt.2014.08.020. PMID: 25196857Free PMC Article

Prognosis

Identification of Germline Non-coding Deletions in XIAP Gene Causing XIAP Deficiency Reveals a Key Promoter Sequence.
Sbihi Z, Tanita K, Bachelet C, Bole C, Jabot-Hanin F, Tores F, Le Loch M, Khodr R, Hoshino A, Lenoir C, Oleastro M, Villa M, Spossito L, Prieto E, Danielian S, Brunet E, Picard C, Taga T, Abdrabou SSMA, Isoda T, Yamada M, Palma A, Kanegane H, Latour S
J Clin Immunol 2022 Apr;42(3):559-571. Epub 2022 Jan 9 doi: 10.1007/s10875-021-01188-z. PMID: 35000057
Inherited chromosomally integrated human herpesvirus-6 in a patient with XIAP deficiency.
Inoue K, Miura H, Hoshino A, Kamiya T, Tanita K, Ohye T, Park MJ, Yanagimachi M, Takagi M, Imai K, Morio T, Yoshikawa T, Kanegane H
Transpl Infect Dis 2020 Oct;22(5):e13331. Epub 2020 Jun 8 doi: 10.1111/tid.13331. PMID: 32424944
Transplantation in rare lymphoproliferative and histiocytic disorders.
Cruz-Chacon A, Mathews J, Ayala E
Cancer Control 2014 Oct;21(4):335-42. doi: 10.1177/107327481402100410. PMID: 25310215Free PMC Article

Clinical prediction guides

Mutations at the C-terminus of CDC42 cause distinct hematopoietic and autoinflammatory disorders.
Coppola S, Insalaco A, Zara E, Di Rocco M, Marafon DP, Spadaro F, Pannone L, Farina L, Pasquini L, Martinelli S, De Benedetti F, Tartaglia M
J Allergy Clin Immunol 2022 Jul;150(1):223-228. Epub 2022 Feb 12 doi: 10.1016/j.jaci.2022.01.024. PMID: 35157921
Identification of Germline Non-coding Deletions in XIAP Gene Causing XIAP Deficiency Reveals a Key Promoter Sequence.
Sbihi Z, Tanita K, Bachelet C, Bole C, Jabot-Hanin F, Tores F, Le Loch M, Khodr R, Hoshino A, Lenoir C, Oleastro M, Villa M, Spossito L, Prieto E, Danielian S, Brunet E, Picard C, Taga T, Abdrabou SSMA, Isoda T, Yamada M, Palma A, Kanegane H, Latour S
J Clin Immunol 2022 Apr;42(3):559-571. Epub 2022 Jan 9 doi: 10.1007/s10875-021-01188-z. PMID: 35000057

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